For the Caregiver: The Influence of Diagnosis on Standards of Care

Editor’s Note: Babette Grzyboski, R.N., is Director of Community Care Services, Alzheimer’s Community Care Association of Palm Beach and Martin Counties, Florida. This article is reprinted with the author’s permission.

In today’s society, as our population of elderly is increasing and living longer, the numbers of those with dementing illnesses is on the rise. Diagnosing dementia disorders will assume greater importance, especially in respect to health care. The chances of caring for someone affected by one of these illnesses will become greater as time goes on.

Dementia is not a disease itself, but a group of symptoms that result from a disease process. There are well over 60 diseases that cause the symptoms of dementia. Historically, the dementing illnesses were lumped into one major category and called Alzheimer's disease or senile dementia. Recent trends have practitioners and researchers searching for definitive diagnoses as each disease has its own unique characteristics. The more we learn to identify each dementia, the better we are able to treat the symptoms and care for those affected. Distinguishing one type from another is also important because it can influence medication usage.

Diffuse Lewy Body Disease (DLB)

In the past 15 years, increasing recognition has been given to this disorder, now thought to be the second most prevalent cause of dementing illness, at between 15 and 25 percent. Onset of Lewy Body Disease is typically between the ages of 60 to 80, with males more at risk. Most cases have been sporadic, and some genetic tendencies are seen. The presence of the APEO-4 gene is considered a risk factor; average duration is 6 to 8 years.

Several identifying traits are seen pathologically on autopsy in Lewy Body Disease. Degeneration of the cortical areas of the brain is apparent. Changes are seen in the substantia nigra, an area of the brain stem. The cells of this area are responsible for making the neurotransmitters dopamine and noradrenaline, therefore deficits occur. With the disease process, these cells die, causing loss of pigment so the area appears pale. The remaining nerve cells contain abnormal structures called Lewy Bodies, the hallmark of this disease. Lewy Bodies are concentric hyaline inclusions within the cytoplasm of neurons.

Clinical presentation assists many practitioners in distinguishing Lewy Body from other dementias. Violent fluctuations in cognitive abilities from day to day may be present. Theoretically, Lewy Body Disease manifests itself with symptoms of both Parkinson's and Alzheimer's. However, initial symptoms usually encompass bradykinesia, rigidity, difficulty initiating movements, frequent falls and syncope or transient loss of consciousness. Behavioral disturbances such as severe hallucinations, delusions and outbursts are common. These symptoms usually occur before a true loss of memory is apparent.

In almost all patients, the disease is relentless and progressive; the dementia becomes global and severe. Eventually, patients become profoundly demented and immobile, and usually succumb to pneumonia or illness after an average of 7 years from the onset of symptoms.

Patients with Lewy Body are at risk of catastrophic results with Haldol and neuroleptic agents. These drugs can cause extrapyramidal signs, even if not present already, that are often prolonged, profound and can be fatal. Aricept is usually beneficial.

Vascular dementia

These dementias account for 10 to 20 percent of all cases. There is an increased incidence in patients over the age of 85 and in African-Americans and Japanese. The most commonly associated risk factors, hypertension, heart disease, diabetes and arteriosclerosis, are present.

Pathological findings are always accompanied by vascular disease. Multi Infarct Dementia is associated with hypertension and arteriosclerosis and presents with several small (lacunar) strokes, usually in the basal ganglia or cerebellum. Some patients will have severe white matter disease, usually indicative of Bins-Wagner's disease. A third very common form of vascular dementia, especially in those over 85, is hippocampal sclerosis. Damage to the hippocampus with lunar infarcts is seen. This form is seen in those individuals who develop dementia after receiving general anesthesia. Given age-associated vascular changes that are very prevalent in this age group, it is clear why the elderly cannot tolerate hypoperfusion that might be well within normal anesthetic limits in a younger adult.

Clinical presentation is concurrent with an abrupt onset and stepping-stone-like course. Punctuated discrete insults are associated with worsening symptoms. Wandering, urinary incontinence, confusion and gait disturbances are all prominent symptoms of vascular dementia. Aricept is not helpful in these cases.

Pick's Disease

Arnold Pick first described Pick's Disease in a 71-year-old man back in 1892. Pick's Disease is a progressive, degenerative dementia of unknown cause that affects the frontal and temporal regions of the brain. Recent studies have shown a strong genetic tendency in certain families. Onset is usually between the ages of 50 to 60. Isolated cases have been documented at the age of 20 and as late as 80. It is often seen more in Europe and in men. The illness usually progresses over a time span of 2 to 15 years. It is the cause of less than 10 percent of the diagnosed dementias; however, recent trends have seen an increase in its diagnosis.

As in Alzheimer's, conclusive diagnosis is only on autopsy after death. The existence of Pick bodies, round argyrophilic intraneural inclusions composed of abnormal Tau proteins, are seen. These bodies disrupt the ability of neurons to work and lead to cell death. Senile plaque formation is usually not seen with Pick's.

Again, characterizing the symptomology is beneficial in the aid of diagnoses. Often, language problems surface early in the disease. The use of generic words, inability to find the right word (instead describing the object or substituting a word), and lack of speech spontaneity are examples of this. Bizarre psychiatric symptoms are also prevalent as a distinguishing feature. Obsessive behavior, ritualistic activities, severe personality disturbances and uninhibited actions are examples of these.

Creutzfeldt-Jakob Disease (CJD)

CJD can affect anyone regardless of sex, ethnic origin, and demographics, but the usual age of onset is from 50 to 75. CJD is a transmissible spongiform encephalopathy. Death is usually inevitable within one year of symptom onset. This disease is thought to be sporadic, inherited or transmitted through exposure. Frequency is estimated at 1:1,000,000.

The cause of CJD is an unconventional infectious pathogen called a PRION (proteinaceous infectious particle). They are theorized to transform normal protein molecules into infectious ones by altering their shape. The familial forms are associated with mutations in the gene coding for the PRION protein. Transmission of CJD has been documented from corneal transplant, implantation of ECG electrodes, neurosurgical procedures and pituitary hormone administration. Additionally, the sterilization procedures used in hospitals are ineffective in decreasing the infectious nature of this pathogen.

Clinical presentation is reflective of an insidious onset with a rapid progression of declining cognition. Onset could show insomnia, depression, confusion, and personality changes. Strange physical sensations and problems with coordination and sight have also been reported. Involuntary jerking movements and severe progressive dementia encompass the final stage of CJD.

Huntington's Disease

Huntington's Disease is a hereditary degenerative disease that is more predominant in men. The pattern of degeneration is linked to a specific mutation in a gene located on chromosome 4. Unfortunately, carrying a single copy of this gene leads to a high probability (50%) of passing it on to offspring. The disease will progress over 10 to 15 years; clinical presentation is severe. Initial signs include movement disorders such as chorea and dyskinesia, facial muscle tremors, and cognitive difficulties. As the disease progresses, behavior problems set in. These are the most difficult symptoms as the patient suffers violent mood swings, aggressive behaviors and depression.

Conclusion

The aforementioned conditions serve as a review of the more common non-treatable degenerating dementias. Many of the listed symptoms are the initial and more predominant ones, and many have subtle differences in presentation. Eventually, in all of these diseases, the patient exhibits classic signs of dementia. Most of these diseases have crossed our paths in working with Alzheimer's patients and those with related disorders. Following the course of the disease helps us understand what to expect. It has made our job of caregiving easier and promoted a better quality of life for our patients.

*second most common cause of degenerative dementia
*onset at 60-80 years of age
*loss of pigment in cells of substantia nigra • presence of Lewy Bodies
*violent fluctuation of cognition
*unsteady gait
*muscle rigidity

Multi-Infarct Dementia

*accounts for 10%-20% of dementia cases
*more prevalent in patients greater than 85 years of age
*associated with vascular risk factors
*presence of vascular disease
*accompanied by several small strokes
*abrupt onset
*stepping stone course
*wandering
*urinary dysfunction

Pick's Disease

*onset at 50-60 years of age
*isolated cases at age 20 years and greater than 80 years of age
*progresses over 2-10 years
*presence of Pick's Bodies
*language difficulties
*bizarre psychiatric symptoms
*ritualistic activities

Creutzfeldt-Jakob Disease

*onset at 50-75 years of age
*transmissible
*results in death within one year
*caused by pathogen called PRION
*insidious onset
*rapid progression
*confusion
*personality changes
*strange physical sensation

Huntington's Disease

*genetic
*progresses over 10-15 years
*mutation of gene on chromosome 4
*degeneration of nuclei in forebrain that control movements and selection of action
*movement disorders
*facial grimacing
*severe behavior problems
*violent mood swings